INTRODUCTION
Congenital lobar emphysema (CLE) also known as congenital lobar
overinflation, is a rare developmental lung malformation. The
respiratory system begins to develop during the third week of pregnancy,
and developmental anomalies during this stage can lead to parenchymal
lung malformations (1-3). CLE is the hyperaeration of one or more lung
lobes due to partial obstruction (4) and occurs at a rate of
1/20,000–30,000 live births. CLE is more common in male infants but
rarely causes respiratory distress in newborns (5-7). The diagnosis can
be performed with ultrasonography at various stages of the pregnancy
(8). Approximately half of the patients with CLE are symptomatic at
birth, and most are diagnosed within the first 6 months of life (9-11).
The most affected lobes are the left upper lobe, right upper lobe, and
right middle lobe. Involvement of the lower lobes and more than one lobe
is very rare (9-13). Pathology samples typically show no tissue damage,
except for acquired emphysema and the occasional accompanying cartilage
hypoplasia. Acinar and alveolar structures are both normal in CLE;
however, the alveoli can experience secondary hypertrophy, and the
affected lung tissue cannot postnatally mature (9). Bronchial collapse
develops due to structural damage in the bronchial wall and can cause
distal air trapping on expiration (14). The etiology of CLE can include
hypoplasia, dysplasia, and the absence of primary or secondary bronchial
cartilage; however, most of these conditions are idiopathic. CLE can be
observed as the result of congenital bronchial diseases such as primary
bronchial stenosis/atresia and bronchomalacia. Secondary causes of CLE
include meconium aspiration and the obstruction of mucous plaques and
membranes (15-17). Although rare, bronchogenic cysts and mediastinal
masses have also been shown to cause CLE (18). The progressive increase
in ventilation can also result in compression of adjacent organs and,
rarely, impaired perfusion. Substernal and subcostal retraction,
wheezing, cyanosis, and feeding difficulties can occur during the
neonatal period, whereas chronic cough and frequent lung infections can
occur during infancy and might be difficult to distinguish from foreign
body aspiration (9).
During bronchial development, as cardiac structures develop between 4
and 6 weeks of pregnancy, CLE can also be accompanied by congenital
heart diseases (CHDs), such as patent ductus arteriosus, atrial septal
defect, ventricular septal defect, total anomalous pulmonary venous
drainage, and tetralogy of Fallot at varying rates. External compression
of vascular structures can sometimes be the cause of secondary CLE (19).
Hyperechogenicity, mediastinal shift, and polyhydramnios can be observed
through intrauterine ultrasonography of the lung structures, with normal
blood flow (20-23). During the postnatal period, the first choice for
diagnosis is chest radiography (CXR), which can reveal increased
aeration in the affected lobe. In severe cases, the CLE might be
accompanied by herniation of the opposite lung (9). Thoracic computed
tomography (CT) performed with contrast provides more detailed
information on vascular compression, endobronchial occlusive masses,
bronchial atresia, etc., in elucidating the causes and differential
diagnosis. Anatomical anomalies such as bronchial atresia,
bronchomalacia, mucus plaques, and mucosal flaps can be observed with
flexible bronchoscopy (FB). Suspected foreign body aspiration can also
be ruled out with FB (23).
Treatment decisions are reached according to clinical, radiological, and
bronchoscopy findings. When the symptoms and signs are mild to moderate,
when the patient’s growth and development are not affected, and when
there is no accompanying respiratory distress, a conservative approach
is adopted. Lobectomy is recommended only for patients with severe and
progressive findings (23). Close follow-up is recommended for patients
for whom a conservative approach is advised, given that the clinical
findings can progress at later ages and that surgery might be required
(5).
The aim of this study is to retrospectively examine the clinical,
radiological, and bronchoscopy findings of patients diagnosed with CLE
in our clinic who are followed up with conservative and/or surgical
approaches and to compare our results with those in the literature.